Scleroderma Heart Disease

Marasini, Bianca; Massarotti, Marco; Cossutta, R.

doi:10.1177/039463200501800401

Cited by 8 publications

(5 citation statements)

References 44 publications

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“…Cardio-myopathy with ventricular diastolic dysfunction and rhythmic disturbances are the most important forms, since they are associated with a very poor prognosis. 6 The disturbed rhythms may be life threatening, with sudden cardiac death reported in some cases of SSc. 7 Increasing evidence strongly suggests that cardiac involvement is related to recurrent focal ischemic injury causing irreversible myocardial fibrosis.…”

Section: Introductionmentioning

confidence: 99%

Autonomic Dysfunction Predicts Early Cardiac Affection in Patients with Systemic Sclerosis

Othman

Assaf

Farouk

et al. 2010

Clin Med�Insights�Arthritis�Musculoskelet Disord

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Objective: To detect the early preclinical alterations in cardiac autonomic control as well as altered cardiac function in systemic sclerosis (SSc) patients and their relevance to the clinical features of the disease using noninvasive methods. Methods: 30 SSc patients and 15 healthy controls matched for age and sex underwent clinical examination, serological analysis, and echocardiographic assessment including Doppler flow imaging to evaluate cardiac function, and 24-hour Holter monitoring analyzed for arrhythmia and heart rate variability (HRV) in the time and frequency domains. Results: The trans-mitral Doppler of early to atrial wave (E/A) ratio was reversed in five patients (16.6%) and the tricuspid E/A ratio was reversed in 10 patients (33.3%). Holter analysis for SSc patients revealed an increased prevalence of premature ventricular contractions (PVC) $ 10/h (P = 0.02), supra-ventricular tachycardias (SVTs) (P = 0.2), and total PVC count (P = 0.0000). Highly significant (P = 0.000) impairment in all HRV parameters was demonstrated in the SSc patients. Total skin thickness score (TSS), Raynaud's phenomenon and anti-scleroderma 70 (anti-SCL70) showed significant positive correlations with all arrhythmia parameters, while showing a significant negative correlation with the impaired ventricular diastolic function and various HRV parameters. No correlation was found between arrhythmia and HRV parameters and disease duration, disease type, or presence of anti-centromere antibodies. Conclusion: Low heart rate variability, increased TSS and the presence of anti-SCL70 are correlated with preclinical cardiac involvement in SSc patients and may predict the likelihood of malignant arrhythmia and sudden cardiac death. Therefore, noninvasive HRV evaluation before clinical cardiac involvement in these patients might be beneficial when added to the clinical and laboratory assessments in detecting high-risk patients, and may allow for implementation of preventive measures and initiation of appropriate therapy early in the course of the disease.

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Section: Introductionmentioning

confidence: 99%

Autonomic Dysfunction Predicts Early Cardiac Affection in Patients with Systemic Sclerosis

Othman

Assaf

Farouk

et al. 2010

Clin Med�Insights�Arthritis�Musculoskelet Disord

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“…Focal distribution of myocardial changes may result in a variation of histological findings from different areas of the heart [5]. Thus, a routine myocardial biopsy may not always find the changes in the myocardium because of patchy involvement in a sclerodermic heart [6].…”

Section: Discussionmentioning

confidence: 99%

Left Ventricular Aneurysm in Patient With Scleroderma

Wulandari¹,

Watanabe

Matsuura³

et al. 2006

J Echocardiogr

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We present a case of a patient with scleroderma with a rare form of cardiac involvement. Transthoracic echocardiography showed a left ventricular (LV) aneurysm in the base of the posterior free-wall. Cardiac catheterization revealed normal coronary arteries with a prominent aneurysm seen on left ventriculogram. Although ventricular dilatation is commonly found in scleroderma, a more serious cardiac complication such as LV aneurysm may also develope. (J Echocardiogr 2006; 4: 123-125) Key words: LV aneurysm, scleroderma symptoms of heart failure while continuing steroid therapy for the scleroderma.

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“…Pericardial involvement is characterized by fibrinous pericarditis and chronic effusion. [ 4 , 5 ] Cardiac tamponade is rare. [ 4 ] Mitral valve prolapse is reported to be the most frequent form of endocardial involvement.…”

Section: Introductionmentioning

confidence: 99%

Clinical courses and predictors of left ventricular systolic dysfunction in systemic sclerosis: A cohort study

Werakiat,

Pussadhamma,

Mahakkanukrauh

et al. 2024

Rheumatology and Immunology Research

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Background and Objectives Left ventricular systolic dysfunction (LVSD) is a cardiac involvement that is the leading cause of death among patients with systemic sclerosis (SSc). We aimed to define the clinical course and predictors of LVSD among SSc patients. Methods We conducted a cohort study among adult patients with SSc who were followed up from 2013 to 2020. Semiparametric Cox regression analysis with robust clustering by cohort identification number was used to evaluate the predictors of LVSD. Results Among the 3, 987 person-years, LVSD was defined in 35 of 419 SSc patients for an incidence of 0.88 per 100 person-years. The median duration of the disease was 8.5 (interquartile range (IQR) 4.9–12.9) years. Every 1-point increase in the modified Rodnan skin score (mRSS) and salt and pepper skin were strong predictors of LVSD, with a respective adjusted hazard ratio (HR) of 1.05 and 3.17. During follow-up, 26 cases (74.3%) had unimproved LVSD. The strong predictors of the unimprovement of LVSD were every 1-point increase in mRSS (HR 1.05), every 1 mg increase in prednisolone treatment (HR 1.05), and every 1 U/L increase in creatine kinase (CK) (HR 1.001). Mycophenolate treatment was a protective factor against the unimprovement of LVSD in SSc (HR 0.15). Conclusions LVSD was frequently found in patients with diffuse cutaneous SSc, and in most cases, it remained unimproved during follow-up. High mRSS, steroid use, and high CK levels were predictors of unimproved LVSD, whereas mycophenolate treatment might prevent the progression of LVSD. Steroids should be prescribed with caution in patients with longer disease duration.

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Scleroderma Heart Disease

Cited by 8 publications

References 44 publications

Autonomic Dysfunction Predicts Early Cardiac Affection in Patients with Systemic Sclerosis

Autonomic Dysfunction Predicts Early Cardiac Affection in Patients with Systemic Sclerosis

Left Ventricular Aneurysm in Patient With Scleroderma

Clinical courses and predictors of left ventricular systolic dysfunction in systemic sclerosis: A cohort study

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